Total small bowel and colon aganglionosis.
A case of total small bowel and colon aganglionosis is presented and 11 previous cases are briefly reviewed. The relatively long survival of our patient in an excellent condition provided the possibility of studying the behaviour of the aganglionic small bowel, disclosing that in the absence of propulsion, absorption is uninvolved and enteral feeding is possible. Aganglionosis of the ileum does not imply absence of muscle activity (leading to misleading bowel sounds and visible peristalsis on X-ray studies), and often the affected small bowel is dilated. In cases of Hirschsprung's disease with small bowel involvement we recommend as routine a full thickness biopsy of the jejunum, to ensure early diagnosis of the fatal disease of total intestinal aganglionosis.
D003106:Colon / Q000002:abnormalities / Q000294:innervation*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007421:Intestine, Small / Q000002:abnormalities / Q000294:innervation*; D007583:Jejunum / Q000473:pathology / Q000601:surgery; D010288:Parenteral Nutrition